X inactivation and disease

X inactivation and disease

X inactivation is the mechanism by which mammals adjust the X-linked gene dosage between the sexes. The dosage difference between XX females and XY males is functionally equalized by silencing one of the two X chromosomes in female cells. This dosage-compensation mechanism is based on the long funct...

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Veröffentlicht in:Seminars in cell & developmental biology 2010-04, Vol.21 (2), p.194-200
Hauptverfasser: Agrelo, Ruben, Wutz, Anton
Format: Artikel
Sprache:eng
Schlagworte:
Animals
Chromatin
Dosage compensation
Dosage Compensation, Genetic - physiology
Epigenetics
Female
Gene regulation
Genetic Diseases, X-Linked - genetics
Humans
Male
RNA, Long Noncoding
RNA, Untranslated - physiology
Stem cells
X Chromosome Inactivation - physiology
X inactivation
Xist RNA
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Beschreibung
Zusammenfassung:X inactivation is the mechanism by which mammals adjust the X-linked gene dosage between the sexes. The dosage difference between XX females and XY males is functionally equalized by silencing one of the two X chromosomes in female cells. This dosage-compensation mechanism is based on the long functional Xist RNA. Here, we review our understanding of dosage compensation and Xist function in the context of disease.
ISSN:1084-9521
1096-3634
DOI:10.1016/j.semcdb.2009.09.017